Case Reports in Immunology
Volume 2014 (2014), Article ID 910215, 7 pages
http://dx.doi.org/10.1155/2014/910215
Case Report
1Department of Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA
2Division of Allergy & Immunology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA
3Division of Allergy & Immunology, Boston Children’s Hospital, Harvard Medical School, Boston, MA 02115, USA
4Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA
5Division of Pulmonary & Critical Care Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA
6Pediatric Allergy & Immunology and the Center for Immunology and Inflammatory Diseases, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA
2Division of Allergy & Immunology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA
3Division of Allergy & Immunology, Boston Children’s Hospital, Harvard Medical School, Boston, MA 02115, USA
4Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA
5Division of Pulmonary & Critical Care Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA
6Pediatric Allergy & Immunology and the Center for Immunology and Inflammatory Diseases, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA
Received 30 May 2014; Revised 15 September 2014; Accepted 20 September 2014; Published 15 October 2014
Academic Editor: Jiri Litzman
Copyright © 2014 Jocelyn R. Farmer et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
Humoral immune deficiencies have been associated with noninfectious disease complications including autoimmune cytopenias and pulmonary disease. Herein we present a patient who underwent splenectomy for autoimmune cytopenias and subsequently was diagnosed with humoral immune deficiency in the context of recurrent infections. Immunoglobulin analysis prior to initiation of intravenous immunoglobulin (IVIG) therapy was notable for low age-matched serum levels of IgA (11 mg/dL), IgG2 (14 mg/L), and IgG4 (5 mg/L) with a preserved total level of IgG. Flow cytometry was remarkable for B cell maturation arrest at the IgM+/IgD+ stage. Selective screening for known primary immune deficiency-causing genetic defects was negative. The disease course was uniquely complicated by the development of pulmonary arteriovenous malformations (AVMs), ultimately requiring bilateral lung transplantation in 2012. This is a patient with humoral immune deficiency that became apparent only after splenectomy, which argues for routine immunologic evaluation prior to vaccination and splenectomy. Lung transplantation is a rare therapeutic endpoint and to our knowledge has never before been described in a patient with humoral immune deficiency for the indication of pulmonary AVMs.
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